In July 2009, our beautiful son Adam was diagnosed with high-risk neuroblastoma, an aggressive children's cancer. Despite four years of treatment in the UK, Germany, and America, Adam sadly passed away on 11th July 2013 at home with his family. Visit Adam's Appeal at http://adamsappeal.org.

Thursday, 12 January 2012

Well it’s the 12th of January and this is my first update of the new year. For which, for once, I make no apologies. Rather I am starting as I mean, and very much hope, to go on. For we have begun 2012 with the best of intentions of making it a transitional year. A year when Adam’s life, and by extension our own, is not entirely dominated by cancer, and all that comes with it. Of course it will still be a massive part of it, and right now I cannot ever foresee a time when it won’t be the single biggest thing in our lives, and that to which we afford the greatest time, effort and worry. And there are no certainties, only fear and uncertainty in perpetuity, which is one of the many things we have to live with.

Adam is doing okay. He completed the fifth and final round of antibodies without any problems, and is currently half-way through his eighth round of 13-cis-retinoic acid. We got back from Germany with everyone, and everything, still intact. The weather driving through Germany and Holland was some of the worst we’ve experienced – high winds and driving rain – unpleasant at the best of times, but worse in a camper van loaded up to the eyeballs and with a luggage box stuck on the back.

Christmas has been declared a success. Whilst it was never going to be an ideal situation we all enjoyed being out in Germany together, and there was only a couple of times when going to the hospital was met with disapproval from one of the children. Towards the end of the second week, when Adam was back at the house on the portable pump, there was a bit of boredom that set in. I was working, the novelty had worn off, there wasn’t a whole lot to do that hadn’t already been done several times before, and there wasn’t the expectant build up to Christmas that carried us through the first week and kept everybody in good spirits.

Adam has an MIBG scan scheduled for next week at the Marsden, and then we travel to Greifswald the following weekend for MRI scan, bone marrow biopsies, and other end-of-treatment tests. It will also be an opportunity to discuss what to do next with the experts over there, though more of that later in this update. He’s been back at school part-time this week again, though he does retain this idea that he can come and go as and when it pleases him. Getting him back into a more disciplined routine in the relation to attending school is going to be something of a challenge. We had a slight misunderstanding earlier in the week about what time he was getting picked up … resulting in me receiving a call to say Adam was sat outside the office crying because nobody has come to collect him, and refusing point blank to return to his classroom. “Before lunch” in Adam’s view means anytime before lunch. Needless to say in future he will come home “when everybody else goes in to have their lunch”, “at the end of second play when everybody else goes back into class”, or “at normal home-time the same as everybody else”.

The upcoming scans might render everything I am about to write entirely meaningless and irrelevant but the new year, along with the completion of antibody therapy, heralds a change in attitude and outlook. We can’t go on forever waiting for the scans that shows Adam’s cancer is growing again, or has spread. Especially if we truly hope and believe there is a chance it may not. Because to do so will be to miss out on or minimize everything that could have been in the meantime, when we should be doing the exact opposite. Upcoming scans whenever they are might render any plans for what comes after entirely meaningless and result in future plans being torn up. That doesn’t mean we shouldn’t have any and instead content ourselves with simply bumping along.

Instead of being a child with cancer who is eight years old, henceforth Adam is an eight year old boy who has cancer. And that means some of the things we have almost entirely neglected over the past two-and-a-half years will no longer be neglected. So we will be concerning ourselves a lot more with Adam’s schooling, about how to integrate his need for education with his need for continued treatment. And we will be thinking a lot more about his need for more social interaction with his peer group; he has expressed an interest in re-starting tennis lessons for example, and why shouldn’t he? He has spent the last 30 months with a central line tunnelled up under his skin, across his shoulder blade, and into a vein in his neck. Do we want to leave it in because it makes weekly blood draws easier? Or because he might need intravenous support or medication? Or so it’s there ready and waiting if the worst happens and we find ourselves back on treatment for relapse or progression? Or rather do we ask to have it removed, and let Adam regain a bit more normality, allow him to bath properly, play football without fear, swim without the aid of a dry suit?

So this year the plan is for a shift in emphasis, a rearrangement of the relative priorities, a move away from cancer and towards ‘normal’. But of course, Adam isn’t a normal. Adam has cancer. Neuroblastoma, a cancer that can strike back at any time, even years after it has seemingly gone for good. It hasn’t gone for good until it’s never come back, and if you wait for that day then you’ve already missed the boat. So these changes are not in any way a declaration that Adam no longer has cancer, or that he’s safe now. He has cancer, he will always have cancer. Nor are they an admission that we are tired of fighting, of doing everything we can to see our little boy grow up. They are precisely because we want to see him grow up, but grow up having had the childhood of a child, with the same sort of opportunities, the same sort of experiences, the same sort of memories. This isn’t going to make our lives easier, it’s going to make it more difficult. We are not stopping treatment, we are looking to move to options that are available orally, that have low toxicity, that we can give to Adam with the dual aim of keeping his disease stable, and affording him the best possible quality of life. Don’t get me wrong, if something comes along that requires intravenous administration, needs us to spend the next year in North America, and is known, or even believed though not yet proven, to be much more effective than anything available orally we’ll be on the next flight out of here. But there are no absolutes in that regard, not at the moment. It so happens that my thinking about treatments, and what are the next best options for Adam, actually came before all this other stuff about trying to reorient our lives a little. It was the fact that those options come in oral form that led us to think more widely about what else it might allow us to do.

So here’s the plan.

First off, we’ll keep doing all the things at home that we have been for the past eighteen months of so in terms of nutrition, supplementation and general wellbeing. And that is a lot. And it’s time consuming. And it’s each and every day. At times it’s a complete and utter arse ache and we’d (and Alison in particular who gets to do most of it) would rather be doing pretty much anything instead. But we feel it’s important, we feel it’s helped to keep Adam strong, helped him bounce back quickly whenever we’ve had difficult periods such as high-dose, or the first cycle of antibodies and subsequent pneumonia. We won’t be sacrificing it, nor compromising on it, not for anything, not for anybody. We’ll be fitting it around other stuff where we can, and where we can’t we’ll be fitting other stuff around it.

Next, Adam is going to remain on 13-cis-retinoic acid until May by which time he will have completed twelve rounds instead of the normal six. The logic behind this is that Adam’s original diagnosis was differentiating neuroblastoma, suggestive of a form that is more predisposed to mature by itself, and 13-cis-RA is designed to promote tumour differentiation (maturation) to a benign form. After that we are hoping that Adam will be able to begin fenretinide, another retinol (vitamin A) derivative that induces programmed cell death (apoptosis). Its mechanism of action is different to retinoic acid (apoptosis versus maturation) and may therefore be effective in targeting cells that do not mature in response to 13-cis-retinoic acid.

So in Adam’s case we would be hoping the 13-cis-RA has done it’s job and there are no more neuroblastoma cells left that haven’t fully matured. If there are cells left that evaded detection, or were not disposed to mature, fenretinide gives us another chance at killing them. At worst (a dichotomy because actually I mean at best) if it's not actually needed (we have no way of knowing) the fenretinide shouldn’t have any severe or lasting adverse side-effects even if there are no neuroblastoma cells to go after. The toxicity is generally quite manageable, and will basically mean continuing the regular blood draws and liver profile tests that we have been doing ever since Adam started 13-cis-RA. There have been Phase I and Phase II studies of fenretinide in children with neuroblastoma in which objective responses have been observed. More recently it has been provided as an oral powder formulation that has increased bioavailability and will, it’s hoped, lead to a better response rate as a consequence. There are other possibilities for continuing treatment, but they are far from abundant. As Adam only has MIBG-positive disease that is not proven by biopsy to be active he is excluded from a large proportion of clinical trials. We could try and take a piece of his thigh bone for biopsy, but it would be highly unlikely to be successful as there are no measurable lesions either by MIBG, or MRI, or PET, or Gallium Octreotide.

One thing I should mention is that we are now getting completely outside the sphere of treatment in the UK. Fenretinide, and indeed all the other options for that matter, are found in America. This may not have been the reason we launched Adam’s Appeal originally – to pay for immunotherapy in America that it turned out Adam couldn’t have as he failed to respond well enough to chemotherapy – but it was the reason we carried on raising money. Our appeal funds now give us the means to pursue the best treatment for Adam regardless of what, or where, it is.

The best and least likely option would be getting fenretinide treatment into the UK. Get it shipped across, take it under the care and guidance of the Marsden. Sounds simple? Yes, it is perfectly simple. And at the same time it is almost certainly impossible. Regulation, safety, bureaucracy, politics, self-interest, jobsworthness. Take your pick. All I know is ‘the system’ collectively is seemingly happy to watch us spend hundreds of thousands on treatment abroad rather than trying to do something to make more treatments available here where they would be open not only to Adam, but also other children suffering from neuroblastoma and running out of options. Option two is to get fenretinide to Greifswald in Germany and use that as the primary institution overseeing treatment, and from where we collect supplies. Right now it seems like a possibility, and certainly more viable than getting it to the UK. However, I’m unsure as to what could be achieved within the necessary timescales. We need to ensure this happens, so any chance it might not is too big a risk to take. And that leaves the third and most likely option, that we turn to America for the treatment. I’ve already had some preliminary discussions and it seems like it might be possible to come up with a workable solution that allows us to remain in the UK, and keeps travel to a minimum both in terms of distance and frequency. It will mean Adam missing some school from time-to-time to travel to the East Coast of America, but I’m sure if it comes to it we can find some positives in such ‘business trips’.

So I hope you are going to be hearing even less from me this year than you did last. Given my penchant for brevity (yeah, right) I’m not sure how this is going to play out. Who knows, it might take me even longer to compose less frequent updates? In the meantime take it as read that from hereon no news is almost certainly good news ...